What is Shy-Drager Syndrome?

Shy-Drager syndrome is probably better known by the name Multiple Systems Atrophy (MSA). It is a rare disorder that creates increasing dysfunction in many of the systems that the body automatically controls (autonomic systems), such as heart rate, blood pressure and sexual function. MSA is not normally diagnosed until people are middle-aged. The disease is progressive, decreasing function of many areas of the body, and ultimately proving fatal. Treatments for it may help control some aspects of the disease but cannot cure it, and as people with this illness are increasingly affected, they lose higher amounts of function in a number of body areas.

The key to curing an illness is often understanding its causal factors, but there is simply not enough known about Shy-Drager syndrome to explain its cause. Researchers don’t see clear genetic or environmental links, though these areas are still under exploration. While cause isn’t easy to understand, progression of MSA is. Parts of the brain begin to atrophy, and as they do so, multiple functions of the autonomic system become impaired.

When Shy-Drager syndrome first is manifested, its symptoms may be variable. Some of the most common early symptoms include loss of sexual function or libido, extreme changes in mood, changes in balance, and poor control of bowels and/or bladder. In addition to these changes, medical exam may find extremely unstable blood pressure that could be too high or too low. This last finding is usually an indication that the condition may be Shy-Drager syndrome.

Most of the symptoms are manifested as the disease progresses. These can include greater impairment in movement, balance, and flexibility, loss of ability to swallow, partial to total speech impairment, and inability for the heart’s electrical system to control heart rhythm. As the condition continues, multiple systems fail, and most often cause of death occurs due to failures in the respiratory system.

While Shy-Drager syndrome doesn’t have a cure, there are medications that support and help prolong greater levels of function. These could include blood pressure medications, some of the same drugs that are used in Parkinson’s like levodopa, medicines for early signs of bladder control issues, and for men, medications to help with erectile dysfunction. Other treatments are considered at later stages. Implanting a pacemaker can regulate heart rhythm, and use of catheters to help with bladder issues becomes common.

Medications can help restore some function and in some cases, drugs may even prolong function slightly. Still, most people with this disorder die within about ten years of its diagnosis because medications don’t address the ongoing atrophy of the brain. The discouraging outlook makes its own urgent argument for continued research on Shy-Drager syndrome.